International Heart Journal
Online ISSN : 1349-3299
Print ISSN : 1349-2365
ISSN-L : 1349-2365
Experimental Studies
Generation of Induced Pluripotent Stem Cells From Patients With Duchenne Muscular Dystrophy and Their Induction to Cardiomyocytes
Akihito HashimotoAtsuhiko T. NaitoJong-Kook LeeRika Kitazume-TaneikeMasamichi ItoToshihiro YamaguchiRyo NakataTomokazu SumidaKatsuki OkadaAkito NakagawaTomoaki HigoYuki KuramotoTaku SakaiKoji TominagaTakeshi OkinagaShigetoyo KogakiKeiichi OzonoShigeru MiyagawaYoshiki SawaYasushi SakataHiroyuki MoritaAkihiro UmezawaIssei Komuro
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Keywords: Cardiomyopathy, Human
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2016 Volume 57 Issue 1 Pages 112-117

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Abstract

Duchenne muscular dystrophy (DMD) is caused by mutations in the DMD gene which encodes dystrophin protein. Dystrophin defect affects cardiac muscle as well as skeletal muscle. Cardiac dysfunction is observed in all patients with DMD over 18 years of age, but there is no curative treatment for DMD cardiomyopathy. To establish novel experimental platforms which reproduce the cardiac phenotype of DMD patients, here we established iPS cell lines from T lymphocytes donated from two DMD patients, with a protocol using Sendai virus vectors. We successfully conducted the differentiation of the DMD patient-specific iPS cells into beating cardiomyocytes. DMD patient-specific iPS cells and iPS cell-derived cardiomyocytes would be a useful in vitro experimental system with which to investigate DMD cardiomyopathy.

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© 2016 by the International Heart Journal Association
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